[Primitive neuroectodermal tumor of the kidney in children]

OBJECTIVES: To characterize primitive neuroectodermal tumor of the kidney as a differential diagnosis for Wilms tumor, and to emphasize the severity of the disease. METHODS: We report the case of a patient with a diagnosis of primitive neuroectodermal tumor of the kidney who underwent nephrectomy and complete tumor resection combined with chemotherapy. RESULTS: Initially, the response of the patient to treatment was favorable, with a few events associated with agranulocytosis resulting from the chemotherapy. Ten months after of the end of treatment, the tumor recurred in the paraspinal cervical region. CONCLUSIONS: Primitive neuroectodermal tumors involving the kidney are very rare and extremely aggressive. In such cases, the pathologist has a major role in establishing a final diagnosis through histological and immunohistochemical methods.